Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

4'-Phosphopantetheine corrects CoA, iron, and dopamine metabolic defects in mammalian models of PKAN.

EMBO molecular medicine - 1 Dec 2019

Jeong Suh Young, Hogarth Penelope, Placzek Andrew, Gregory Allison M, Fox Rachel, Zhen Dolly, Hamada Jeffrey, van der Zwaag Marianne, Lambrechts Roald, Jin Haihong, Nilsen Aaron, Cobb Jared, Pham Thao, Gray Nora, Ralle Martina, Duffy Megan, Schwanemann Leila, Rai Puneet, Freed Alison, Wakeman Katrina, Woltjer Randall L, Sibon Ody Cm, Hayflick Susan J

Abstract excerpt

Pantothenate kinase-associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack of a good mammalian model has impeded studies of pathogenesis and development of rational therapeutics. We took a new approach to investigating an existing mouse mutant of Pank2 and found that isolating the disease-vulnerable brain revealed regional...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Biomarkers
Share to Coenzyme A

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Dopamine

Share to Genotype

Share to Pantothenate Kinase-Associated Neurodegeneration

Share to Phosphotransferases (Alcohol Group Acceptor)

Source

Open in PubMed Open full text

Identifiers

PMID: 31660701
DOI: 10.15252/emmm.201910489
PMCID: PMC6895607

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

4'-Phosphopantetheine corrects CoA, iron, and dopamine metabolic defects in mammalian models of PKAN.PMID 31660701Read ↗

Redesigning therapies for pantothenate kinase-associated neurodegeneration.Semantic proximity Read ↗

Down Regulation Of The Expression Of Mitochondrial Phosphopantetheinyl-Proteins In Pantothenate Kinase-Associated Neurodegeneration: Pathophysiological Consequences and Therapeutic PerspectivesSemantic proximity Read ↗

Pantethine treatment is effective in recovering the disease phenotype induced by ketogenic diet in a pantothenate kinase-associated neurodegeneration mouse model.Semantic proximity Read ↗

Alpha-Lipoic acid supplementation corrects pathological alterations in cellular models of pantothenate kinase-associated neurodegeneration with residual PANK2 expression levelsSemantic proximity Read ↗

Patient-Derived Cellular Models for Polytarget Precision Medicine in Pantothenate Kinase-Associated NeurodegenerationSemantic proximity Read ↗

iPSC-derived neuronal models of PANK2-associated neurodegeneration reveal mitochondrial dysfunction contributing to early disease.Semantic proximity Read ↗

Pantothenate Rescues Iron Accumulation in Pantothenate Kinase-Associated Neurodegeneration Depending on the Type of Mutation.Semantic proximity Read ↗

Unraveling the Hallervorden-Spatz syndrome: pantothenate kinase-associated neurodegeneration is the name.Semantic proximity Read ↗

A Potential Citrate Shunt in Erythrocytes of PKAN Patients Caused by Mutations in Pantothenate Kinase 2.Semantic proximity Read ↗

Pantethine rescues a Drosophila model for pantothenate kinase-associated neurodegeneration.Semantic proximity Read ↗

Therapeutic approach with commercial supplements for pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels.Semantic proximity Read ↗

Pantethine and Neurodegeneration: A Coenzyme A-Centered Framework Linking Metabolism, Neuroinflammation, and Mitochondrial DysfunctionSemantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.